Hypertension In Interstitial Lung Disease Research Articles

What Is the Incidence of Pulmonary Hypertension in Interstitial Lung Disease? How to Manage the Treatment? Is There Any Effect of Pulmonary Hypertension on Prognosis?

What Is the Incidence of Pulmonary Hypertension in Interstitial Lung Disease? How to Manage the Treatment? Is There Any Effect of Pulmonary Hypertension on Prognosis?

Distinguishing Pulmonary Hypertension in Interstitial Lung Disease by Ventilation and Perfusion Defects Measured by Cardiopulmonary Exercise Testing

Background: Pulmonary hypertension (PH) is common in interstitial lung disease (ILD). Since cardiopulmonary exercise testing (CPET) is useful in understanding the pathophysiology of respiratory disorders and can distinguish between ventilation and perfusion (V/Q) defects, it may have a role in the detection of PH in ILD. We evaluated whether CPET can detect PH through analysis of V/Q defects in ILD. Objectives: We aimed to use CPET to determine if there are changes in the ventilation and the activity pattern of mixed-expired carbon dioxide pressure (PE<smlcap>CO</smlcap>₂) and end-tidal carbon dioxide pressure (Pet<smlcap>CO</smlcap>₂) in ILD patients with and without PH. Methods: A retrospective chart review was done of all patients who received lung transplants at the Columbia University Medical Center between 2000 and 2011 with the diagnosis of ILD. CPETs were performed during the 2 years prior to transplantation; right heart catheterizations and pulmonary function tests were performed within 4 months of CPET. Results: The ILD patients with PH demonstrated significantly lower Pet<smlcap>CO</smlcap>₂ and PE<smlcap>CO</smlcap>₂ during certain levels of exercise with a distinctive activity pattern for PE<smlcap>CO</smlcap>₂/Pet<smlcap>CO</smlcap>₂. Conclusions: Evaluation of V/Q defects through the PE<smlcap>CO</smlcap>₂ and Pet<smlcap>CO</smlcap>₂ patterns on CPET in ILD patients can distinguish between patients with and without PH.

Treatment of pulmonary hypertension in interstitial lung disease: do not throw out the baby with the bath water

Pulmonary hypertension (PH), group III of the international aetiological classification [1], is a frequent and severe complication of interstitial lung diseases (ILDs), especially idiopathic pulmonary fibrosis (IPF), the syndrome of combined pulmonary fibrosis and emphysema, ILD associated with connective tissue disease, sarcoidosis, pulmonary Langerhans cell histiocytosis and, rarely, other ILDs [2–6]. Although its prevalence varies widely between these conditions, PH, when present, dramatically impacts morbidity and survival. Despite recent progress with pirfenidone and nintedanib, which reduce the rate of decline in lung function in patients with mild-to-moderate disease [7, 8], management of IPF remains largely supportive, with a relentless progression to respiratory failure and death after a median of only 3 years from the time of diagnosis. Precapillary PH is common in advanced IPF, with a prevalence of 32–46% [9–12] at right heart catheterisation (RHC) at the time of evaluation for lung transplantation. The haemodynamic severity of PH in this context is usually mild, although 2–10% of patients have severe PH with a mean pulmonary artery pressure (mPAP) greater than 35–40 mmHg [9, 10]. PH is associated with increased dyspnoea, decreased exercise capacity as measured by the 6-min walk distance (6MWD) and the peak oxygen uptake at cardiopulmonary exercise testing [9, 13, 14], lower diffusing capacity of the lung for carbon monoxide ( D LCO), greater oxygen requirements and reduced survival [2, 9]. In one study, the 1-year mortality of patients with IPF and associated PH at RHC was 28% versus only 5.5% in those without PH [9]. In subjects with moderate functional impairment, the prevalence of PH is lower and varies depending upon the procedures used. In a recent series of 101 patients who underwent …

Diagnostic and prognostic role of biomarkers for pulmonary hypertension in interstitial lung disease

Pulmonary hypertension (PH) is an important complication to interstitial lung disease (ILD). The aim of the present study was to investigate the relation of NT-proBNP, fibrin D-dimer, troponin-T, uric acid and exhaled nitric oxide (NO) to the presence of PH and mortality in ILD. In a previously described cohort of 212 ILD patients of whom 29 had PH, levels of the above mentioned biomarkers were analyzed as routine tests. A value of NT-proBNP below 95ng/l had a negative predictive value for PH of 99% (95% CI: 94-100). Values of troponin-T were higher in patients with PH (median (inter quartile range)=9 (9-20) vs. 9(9-10)ng/l), and the odds ratio (OR) for PH was increased in patients with abnormal levels of uric acid (OR (95% CI)=3.1(1.1-8.8)). NT-proBNP and troponin-T values above the 50(th) percentile, and uric acid and fibrin D-dimer values above the 90th percentile were each associated with increased mortality. A value of NT-proBNP below 95ng/l may be used as a rule-out test for PH in ILD, while an abnormal value of uric acid is a risk factor for PH. NT-proBNP, troponin-T, uric acid and fibrin D-dimer have prognostic value in ILD patients, while exhaled levels of NO do not seem to predict PH or mortality.

Pulmonary hypertension in interstitial lung disease: Prevalence, prognosis and 6 min walk test

Pulmonary hypertension (PH) is an important complication to interstitial lung disease (ILD). The aim of the present study was to investigate the prevalence and impact of PH on prognosis and exercise capacity in ILD patients. 212 ILD patients were screened for PH by echocardiography. Criteria for PH were either a tricuspid pressure regurgitation gradient >40 mmHg, a tricuspid annular plane systolic excursion <1.8 cm or right ventricular dilatation. If possible, PH was confirmed by right heart catheterisation. Pulmonary function tests and 6 min walk tests (6MWT) were performed. 29 patients (14%) had PH, 16 (8%) had mild and 13 (6%) had severe PH (mean pulmonary artery pressure ≥ 35 mmHg). Compared to patients without PH, lung function parameters were lower in PH patients, a larger proportion had idiopathic pulmonary fibrosis (IPF) (41 vs 21%, p = 0.006), and the hazard ratio for death was 8.5 (95% CI: 4-17). After correction for lung function parameters and the presence of IPF, 6MWT was significantly lower in patients with PH compared to non-PH patients (difference ± SEM: 58 ± 22 m, p = 0.01). PH occurred in 14% of a cohort of patients with ILD and was associated to IPF and lower lung function parameters. Mortality was markedly higher in PH patients, and the presence of PH reduced 6MWT independently of lung function and the presence of IPF. The present results emphasize the need for intensified treatment of patients with ILD and PH.

CT Detection of Pulmonary Hypertension in Interstitial Lung Disease: The Glass Is Half Full

HomeRadiologyVol. 260, No. 3 PreviousNext Reviews and CommentaryEditorialCT Detection of Pulmonary Hypertension in Interstitial Lung Disease: The Glass Is Half FullAthol Wells Athol Wells Author AffiliationsFrom the Interstitial Lung Disease Unit, Royal Brompton Hospital, Sydney Street, London SW3 6NP, England.Address correspondence to the author (e-mail: [email protected]).Athol Wells Published Online:Sep 1 2011https://doi.org/10.1148/radiol.11111251MoreSectionsFull textPDF ToolsImage ViewerAdd to favoritesCiteTrack CitationsPermissionsReprints ShareShare onFacebookTwitterLinked In AbstractThin-section CT is not a consistently reliable surrogate for right-sided heart catheterization in interstitial lung disease, but this conclusion does not in any way detract from other possible applications of thin-section CT in this field.References1 Hansell DM. Small airways diseases: detection and insights with computed tomography. Eur Respir J 2001;17(6):1294–1313. Crossref, Medline, Google Scholar2 Corte TJ, Wort SJ, Gatzoulis MA, Macdonald P, Hansell DM, Wells AU. Pulmonary vascular resistance predicts early mortality in patients with diffuse fibrotic lung disease and suspected pulmonary hypertension. Thorax 2009;64(10):883–888. Crossref, Medline, Google Scholar3 Zisman DA, Karlamangla AS, Ross DJet al.. High-resolution chest CT findings do not predict the presence of pulmonary hypertension in advanced idiopathic pulmonary fibrosis. Chest 2007;132(3):773–779. Crossref, Medline, Google Scholar4 Devaraj A, Wells AU, Meister MG, Corte TJ, Hansell DM. The effect of diffuse pulmonary fibrosis on the reliability of CT signs of pulmonary hypertension. Radiology 2008;249(3):1042–1049. Link, Google Scholar5 Baughman RP, Engel PJ, Meyer CA, Barrett AB, Lower EE. Pulmonary hypertension in sarcoidosis. Sarcoidosis Vasc Diffuse Lung Dis 2006;23(2):108–116. Medline, Google Scholar6 Maher TM, Wells AU. Idiopathic pulmonary fibrosis-related pulmonary hypertension; an exercising diagnosis? Respirology 2011;16(3):381–383. Crossref, Medline, Google Scholar7 Alhamad EH, Al-Boukai AA, Al-Kassimi FAet al.. Prediction of pulmonary hypertension in patients with or without interstitial lung disease: reliability of CT findings. Radiology 2011;260(3):875–883. Link, Google Scholar8 Boerrigter B, Mauritz GJ, Marcus JTet al.. Progressive dilatation of the main pulmonary artery is a characteristic of pulmonary arterial hypertension and is not related to changes in pressure. Chest 2010;138(6):1395–1401. Crossref, Medline, Google Scholar9 Raeside DA, Chalmers G, Clelland J, Madhok R, Peacock AJ. Pulmonary artery pressure variation in patients with connective tissue disease: 24 hour ambulatory pulmonary artery pressure monitoring. Thorax 1998;53:857–862. Crossref, Medline, Google Scholar10 Bossuyt PM, Reitsma JB, Bruns DEet al.. Towards complete and accurate reporting of studies of diagnostic accuracy: the STARD initiative. AJR Am J Roentgenol 2003;181(1):51–55. Crossref, Medline, Google Scholar11 Devaraj A, Wells AU, Meister MG, Loebinger MR, Wilson R, Hansell DM. Pulmonary hypertension in patients with bronchiectasis: prognostic significance of CT signs. AJR Am J Roentgenol 2011;196(6):1300–1304. Crossref, Medline, Google Scholar12 Devaraj A, Wells AU, Meister MG, Corte TJ, Wort SJ, Hansell DM. Detection of pulmonary hypertension with multidetector CT and echocardiography alone and in combination. Radiology 2010;254(2):609–616. Link, Google Scholar13 Raghu G, Collard HR, Egan JJet al.. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis—evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011;183(6):788–824. Crossref, Medline, Google ScholarArticle HistoryReceived June 16, 2011; accepted June 16; final version accepted June 20.Published online: Sept 2011Published in print: Sept 2011 FiguresReferencesRelatedDetailsCited ByA Possible Anatomic Explanation for Inaccuracies in the Derivation of Pulmonary Artery Pressure, with Thin-Section CT, in Interstitial Lung FibrosisEric N. C. 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Pulmonary Hypertension in Interstitial Lung Disease

Pulmonary hypertension (PH) is an often devastating comorbidity in patients with interstitial lung disease (ILD). The prevalence of PH varies widely across the spectrum of ILDs, and it is thought that there are common pathobiological processes, which contribute to both progressive interstitial fibro

Interstitielle Lungenerkrankungen und pulmonale Hypertonie

The prevalence of pulmonary hypertension in interstitial lung disease (ILD) is high (30-40%). However, diagnosis of pulmonary hypertension in ILD is often delayed. Pulmonary hypertension can occur in the absence of advanced pulmonary dysfunction or severe hypoxia and is associated with a worse prognosis. A number of pathogenic mechanisms such as oxidative stress, cytokines, and the endothelin system have been implicated in remodeling of both the lung parenchyma and the vessels. In addition, hypoxic vasoconstriction, vascular destruction and progressive fibrosis play an important role. Since clinical signs are often non-specific echocardiography, radiology and laboratory parameters such as NT-proBNP may be helpful. However, the definitive diagnosis of pulmonary hypertension is still confirmed by right heart catheterization. Treatment options of pulmonary hypertension in ILD are limited to the treatment of the underlying diseases. Newer vasodilating drugs may improve the prognosis but have first to be evaluated in clinical trials. Lung or lung and heart transplantation is the therapeutic option in end stage disease.

Pulmonary hypertension in interstitial lung disease

To examine recent advances in the understanding of the prevalence, pathophysiology, natural history, diagnosis, and treatment of pulmonary hypertension associated with interstitial lung disease. Significant progress has recently been made in understanding the etiology of various causes of pulmonary hypertension, including those associated with interstitial lung disease, and new data regarding the various pathophysiologic mechanisms of pulmonary hypertension in interstitial lung disease have been published. Therapeutic agents that were initially studied in patients with idiopathic pulmonary arterial hypertension are now being applied to interstitial lung disease-related pulmonary hypertension. Short-term hemodynamic benefits and disease-related quality of life outcomes that are associated with these advanced therapies are now better understood, but data regarding long-term outcomes and the efficacy of combination therapies remain lacking for interstitial lung disease-related pulmonary hypertension. Lung transplantation outcomes in the treatment of pulmonary hypertension associated with interstitial lung disease have been further defined. Pulmonary hypertension associated with interstitial lung disease is now frequently recognized and is associated with substantial morbidity and mortality. Various new pharmacotherapies as well as lung transplantation are increasing the treatment options for these disorders, although timing of advanced therapies and long-term benefits and toxicities remain uncertain.

Pulmonary hypertension in interstitial lung disease

In the lungs, parenchymal and vascular remodelling share pathomechanisms that may explain the relatively high prevalence (30-40%) of pulmonary hypertension (PH) in interstitial lung disease (ILD) patients. Notably, PH significantly contributes to exercise limitation and dismal prognosis of ILD patients. The absence of specific clinical symptoms commonly leads to delayed diagnosis. Besides clinical judgment and out-of-proportion reduction in diffusing capacity, severe hypoxaemia or exercise oxygen desaturation, echocardiography and biomarkers such as B-type natriuretic peptide (BNP) and N-terminal pro-hormone BNP are potentially helpful tools in identifying PH. However, right heart catheterisation is still necessary to confirm the diagnosis. Management of PH in ILD comprises treatment of the underlying disease process and long-term oxygen therapy. Affected patients should be listed for lung transplantation without delay, when appropriate. However, due to age and comorbidities only a minority of ILD patients will be eligible for lung transplantation. In the absence of satisfactory therapies for many ILDs, and considering the clinical burden of PH in affected patients, specific vasomodulatory therapies presently approved for PAH may be promising options for ILD patients. Consequently, there is an urgent need for adequately designed clinical trials to assess the effectiveness of specific PH therapy in the context of ILDs.

Pulmonary hypertension in interstitial lung disease

Pulmonary hypertension (PH) may complicate the course of many forms of advanced interstitial lung disease (ILD) and has been shown to portend a worse outcome. The aetiology of PH is likely multifactorial with variable contribution of factors amongst the different diseases. The most common such conditions include idiopathic pulmonary fibrosis, sarcoidosis, connective tissue disease-related ILD and pulmonary langerhans cell histiocytosis. Whether the course and impact of PH in these conditions can be modified by therapy requires further study.

Pulmonary hypertension in interstitial lung disease

Exercise impairment and pulmonary hypertension are common features of interstitial lung disease. Antifibrotic therapies for interstitial lung disease remain unproved; therefore, some interest has been focused on treating the pulmonary vascular impairment these diseases. Patients with pulmonary hypertension secondary to idiopathic pulmonary fibrosis may have normal resting pulmonary artery pressure, but it often rises with exercise. Exercise impairment in idiopathic pulmonary fibrosis has a strong correlation with the degree of pulmonary artery pressure elevation, and hypoxemia, a hallmark of pulmonary hypertension, strongly correlates with survival. Small case series have shown that some patients improve on receiving therapy for pulmonary hypertension secondary to interstitial lung disease. These findings suggest that larger treatment trials for medications targeting pulmonary hypertension in interstitial lung disease are warranted.

The Risk for Hemorrhage from Bronchoscopic Lung Biopsy Due to Pulmonary Hypertension in Interstitial Lung Disease

The Risk for Hemorrhage from Bronchoscopic Lung Biopsy Due to Pulmonary Hypertension in Interstitial Lung Disease